Bilateral hearing loss preceding rhomboencephalitis - a hint for Kelch-like 11 syndrome.

David KrivitskiYifat AlcalayMichael PeerYael ParanOrna EisensteinTima DavidsonAvi Gadoth

Published in: Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology (2022)

Patients presenting with progressive sensorineural hearing loss, vertigo, and ataxia should be evaluated for KLHL11 paraneoplastic syndrome. Furthermore, we support a strong association between anti-KLH11 rhombencephalitis and an underlying seminoma and recommend a thorough search for an undiagnosed germ cell tumor in these patients.

Keyphrases

germ cell
end stage renal disease
case report
ejection fraction
newly diagnosed
hearing loss
multiple sclerosis
chronic kidney disease
peritoneal dialysis
early onset
patient reported outcomes